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Published online before print October 1, 2008, doi:10.1212/01.wnl.0000323811.42080.a4)
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 Volume 71, Number 21, November 18, 2008
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NEUROLOGY 2008;71:1691-1695
© 2008 American Academy of Neurology

Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome

S. Kuwabara, MD, S. Misawa, MD, K. Kanai, MD, Y. Suzuki, MD, Y. Kikkawa, MD, S. Sawai, MD, T. Hattori, MD, M. Nishimura, MD and C. Nakaseko, MD

From the Departments of Neurology (S.K., S.M., K.K., Y.K., S.S., T.H.), Public Health (Dr. Suzuki), and Hematology (M.N., C.N.), Graduate School of Medicine, Chiba University, Chiba, Japan.

Address correspondence and reprint requests to Dr. Satoshi Kuwabara, Department of Neurology, Graduate School of Medicine, Chiba University, 1-8-1, Inohana, Chuo-ku, Chiba, 260-8670 Japan kuwabara-s{at}faculty.chiba-u.jp.

Background: Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a rare multisystem disorder associated with plasma cell dyscrasia. There is increasing evidence that high-dose chemotherapy with autologous peripheral blood stem cell transplantation (Auto-PBSCT) is an efficacious treatment.

Objective: To elucidate the extent and time course of neurologic improvement after Auto-PBSCT in patients with POEMS syndrome.

Methods: Clinical and electrophysiologic findings in nine patients were reviewed. The median follow-up period was 20 months (range, 8 to 49 months). Serum levels of vascular endothelial growth factor (VEGF) were measured by ELISA.

Results: Serum VEGF levels rapidly decreased a month after Auto-PBSCT. Within 3 months, neurologic improvement began, and all the patients showed substantial neurologic recovery during the next 3 months. Particularly, three initially chairbound patients regained ability to walk at 6 months. Nerve conduction studies showed significant increases in conduction velocities and amplitudes within 6 months of treatment. At the end of follow-up periods, neuropathy was still improving, and no patients had recurrence of symptoms.

Conclusion: Autologous peripheral blood stem cell transplantation results in obvious neurologic improvement within 6 months, presumably by extensive axonal regeneration and remyelination. This therapy could be considered as a first line treatment for patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes syndrome with younger onset even if they are tetraplegic.

Abbreviations: Auto-PBSCT = autologous peripheral blood stem cell transplantation; CMAP = compound muscle action potentials; MCV = motor conduction velocities; POEMS = polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes; VEGF = vascular endothelial growth factor.

Editorial, page 1658

e-Pub ahead of print on October 1, 2008, at www.neurology.org.

Supported in part by a grant for research on intractable diseases from the Ministry of Health, Labour and Welfare of Japan (S.K.).

Disclosure: The authors report no disclosures.

Received January 10, 2008. Accepted in final form May 5, 2008.


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C. Chalk
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Correspondence:

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Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome
Mario Sabatelli, et al.
Neurology Online, 6 Feb 2009 [Full text]
Reply from the authors
Satoshi Kuwabara, et al.
Neurology Online, 6 Feb 2009 [Full text]

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